The Failing Right Ventricle in Congenital Heart Disease

In congenital heart disease (CHD) the right ventricle (RV) dons a variety of hats. It might be situated in the normal subpulmonary position and function as half of a 2-pump circulation, but nonetheless be subjected to abnormal loading conditions, surgical interventions, or electrical disturbance. Alternatively, it can be required to generate systemic cardiac output; either in the context of a univentricular circulation or else in concert with a subpulmonary left ventricle. Each role carries different hemodynamic stresses and physiologic demands and the long-term capacity of the RV to withstand the stresses and meet those demands has become recognized as a key contributor to late clinical outcomes. RV failure in CHD usually has an etiology and pathophysiology quite different from RV failure in other circumstances and there is little evidence to support direct transition of treatments well established in acquired heart failure in this patient group. In this review we discuss the requirements for normal RV function, situations in CHD in which the RV is prone to failure, the pathophysiology of RV dysfunction, and potential therapeutic options.